Granulocytic sarcoma in children with acute myeloblastic leukemia and t(8;21)

Background. Granulocytic sarcomas (CS) have been associated with t(8;21). The prognosis of patients with CS is generally regarded as being less favorable than of patients with acute myeloblastic leukemia (AML). CS occurs relatively commonly in Africa and has been reported to affect 10-25% of black children presenting with AML. We sought to establish the incidence of CS in our pediatric population, to determine whether an association with t(8;21) existed, and to report on the outcome of these cases in a single series. Procedure. The records of consecutive pediatric patients treated for de novo AML in Johannesburg between January 1985-December 1995 were reviewed. Fifteen cases of CS among a total of 88 cases of AML presented to the Paediatric Haematology/ Oncology Clinics of the Johannesburg and Baragwanath Hospitals. Fourteen (93%) of these patients were black male children. Results. All 9 cases of orbital GS (60%) and almost all cases with concurrent AML M2 had t(8;21). This translocation was present in only 4 (8.5%) of the remaining 47 AML cases without CS for which cytogenetic data were available. One case presented with a complex chromosomal translocation not previously associated with CS. The median disease-free survival of the CIS patients, using conventional chemotherapy treatment protocols, was significantly better than for the patients with AML and no CS (P = 0.0004). Conclusions. Our data support a strong association between orbital CS, t(8;21), and AML M2 in the pediatric population. This entity occurred virtually exclusively in black male children at presentation. One third of these children who presented with AML had a CS. The favorable prognosis noted in our GS patients on standard induction and intensification therapy without local irradiation conflicts with some previous reports but is consistent with the favorable outcome documented in AML with t(8;21). Med. Pediatr. Oncol. 31: 144-149, 1998. (C) 1998 Wiley-Liss, Inc.